This webpage contains legacy information. The product is either no longer available from the supplier or has been delisted at Labome.
product summary
company name :
Invitrogen
other brands :
NeoMarkers, Lab Vision, Endogen, Pierce, BioSource International, Zymed Laboratories, Caltag, Molecular Probes, Research Genetics, Life Technologies, Applied Biosystems, GIBCO BRL, ABgene, Dynal, Affinity BioReagents, Nunc, Invitrogen, NatuTec, Oxoid, Richard-Allan Scientific, Arcturus, Perseptive Biosystems, Proxeon, eBioscience
product type :
antibody
product name :
BCKDK Monoclonal Antibody (OTI11C9)
catalog :
MA5-24992
quantity :
100 uL
price :
US 514.00
clonality :
monoclonal
host :
mouse
conjugate :
nonconjugated
clone name :
OTI11C9

The same clone is also sold as:
reactivity :
human
application :
western blot, immunohistochemistry, immunocytochemistry, immunohistochemistry - paraffin section
product information
Product Type :
Antibody
Product Name :
BCKDK Monoclonal Antibody (OTI11C9)
Catalog # :
MA5-24992
Quantity :
100 uL
Price :
US 514.00
Clonality :
Monoclonal
Purity :
Affinity Chromatography
Host :
Mouse
Reactivity :
Human
Applications :
Immunocytochemistry: 1:100, Immunohistochemistry (Paraffin): 1:50, Western Blot: 1:2,000
Species :
Human
Clone :
OTI11C9
Isotype :
IgG1
Storage :
-20 C, Avoid Freeze/Thaw Cycles
Description :
The second major step in the catabolism of the branched-chain amino acids, isoleucine, leucine, and valine, is irreversibly catalyzed by the branched-chain alpha-keto acid dehydrogenase complex (BCKD), an inner-mitochondrial enzyme complex composed of 3 catalytic components: a branched-chain alpha-keto acid decarboxylase (E1), a dihydrolipoyl transacylase (E2), and a dihydrolipoamide dehydrogenase (E3). The complex also contains 2 enzymes that regulated the state of activity of the BCKD complex: a kinase (BCKDK), and a phosphorylase. The ubiquitously expressed kinase contains 1 histidine kinase domain. Maple syrup urine disease (MSUD) is a pathology secondary to an enzyme defect in the catabolic pathway of leucine, isoleucine, and valine. Accumulation of these amino acids and their corresponding keto acids results in encephalopathy and progressive neurodegeneration in infants not treated for MSUD.
Immunogen :
Full length human recombinant protein of BCKDK produced in HEK293T cell
Format :
Liquid
Applications w/Dilutions :
Immunocytochemistry: 1:100, Immunohistochemistry (Paraffin): 1:50, Western Blot: 1:2,000
Aliases :
[3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial; AI327402; BCKDHKIN; BCKDK; BCKDKD; BCKD-kinase; BDK; branched chain alpha-ketoacid dehydrogenase kinase; branched chain ketoacid dehydrogenase kinase; branched-chain alpha-ketoacid dehydrogenase kinase; EC 2.7.11.4
company information
Invitrogen
Thermo Fisher Scientific
81 Wyman Street
Waltham, MA USA 02451
https://www.thermofisher.com
800-678-5599
headquarters: USA